Monday, November 13, 2017

Week 221: For real

The cough that both kids have had for more than a week is starting to gradually subside. We've been doing three treatments a day for basically two weeks running but (knock on wood) have not felt like we need to start another course of Cayston yet. We are still relying on Lime as our canary in the coal mine--if he's still coughing then the fact that Lemon is coughing too seems less troubling.
I don't usually dwell on this particular topic on the blog, but this cold has brought with it a lot of puking. Even Lime has coughed to that point a few times with this particular bug, and with Lemon every meal and tube feed has been fraught with peril. Ick. Lots of gross laundry, many, many outings with my new steam mop, etc. But, on the plus side, our issue from a few weeks ago of a certain someone performing bodily functions where they should not be performed seems to have stopped of its own accord. So, although there was gross laundry, there was a dearth of other gross messes to be cleaned, which felt like progress.

However. There is always a "however." However, it seems that all this puking from having a cold and a bad cough and swallowing a lot of mucus gave Lemon a new idea. Perhaps he found that being the potty bandit did not result in the amount or caliber of attention that he had in mind. He certainly noticed that if you puke, your parents tend to sort of rush around, act extremely sympathetic, cater to your needs and wants, etc etc. Those of you familiar with kid logic may see where this is going. We had a few puke incidents that came without the usual warning signs, but sometimes there are no warning signs so I didn't think anything of it. Then we had one last night at dinner that seemed really sort of weird, like everything was 100% fine one minute and 100% the opposite of fine the next, where I actually said to Papa Bear afterwards that I suspected Lemon had done it intentionally. But, we both rejected the idea, because really, who does that.

Then, there was this morning at 4 a.m., when Lemon started yelling "Hello!" very cheerfully from his room. He has a stoplight clock, and he knows that he is not supposed to wake us up when the light is red unless he needs help. We'd had a couple of mornings over the past week or two where he's done this "Hello!" routine at ungodly hours and been given a stern reminder about the whole red light concept. So, I stumbled out of bed and over to his room grouchy and annoyed and ready to give the whole red light speech again. But, when I opened his door, he said, with a big grin, "Look, I threw up!" Having no choice, I sprang into action with the whole cleaning routine, while talking to him in a neutral tone about what was going on. Was he feeling sick? No. Was he coughing a lot? No. What happened? He wanted to throw up, so he did. Of course, by this time, all the commotion had woken Lime up, so we all got to start our day, which I suspect was Lemon's objective all along. 

I sincerely hope this is a short phase. Or maybe just a one-off. Throwing up isn't fun, right? No amount of getting to sit on top of your grumpy mom and discuss the relative merits of various sports cars at 4:41 a.m. is worth that, is it? 

In less gross news, we are still trying to figure out a solution to our overpriced ursodiol problem. We transferred all of our prescriptions from CVS to the children's hospital, and at least there they are only charging us $60 a bottle for the stuff, rather than $90, but $60 is still quite a bit more than the $5 we would like to be paying given that it's a generic medication. We'd like to switch to pills, which we could just crush and give through Lemon's G tube, so I've been in communication with the GI doc's office about that. Well, actually, I have been locked in a MyChart messaging war with a nurse who, how shall I say this, DOES NOT KNOW HOW TO DO MATH. Like, the kind of math you would need to be able to do to divide the number of milligrams in a pill by two and compare the result with the number 170 milligrams to determine if it were a) the same number, b) a smaller number, or c) a larger number, and to make a therapeutic decision on the basis of that comparison. For the moment, I am giving up on this war, because I lack the time and energy. We will just pay for this month and next month's bottles of the compounded stuff, and then see how Lemon's liver enzymes look in December. If the ursodiol seems to be doing something (in which case we will continue it long-term), perhaps I will be able to find a way to actually speak to the doctor, who presumably understands both math and the concept of therapeutic range, and we can work out an actual solution.

A closing note from the "height of irony" department. Lemon's Pulmozyme prescription is filled by CVS specialty pharmacy. Theoretically, it is possible to get the prescription set up to auto-refill. I say theoretically, because Papa Bear is among the most intelligent and diligent of pharmacy customers, and for over a year he has been unable to achieve this despite innumerable attempts. Finally, today, he succeeded. And, tomorrow, he will have to call and transfer the prescription to our new pharmacy. Victory!

Monday, November 6, 2017

Week 220: Changing time

We have certainly come a long way from where we were a year ago. Halloween reminded us that at this time last year, we were doing home IVs, and Lemon in fact had to go trick-or-treating wearing a backpack containing his IV pump. This year, I kept alive the "trick" in trick-or-treat by bringing the kids to get their annual flu shots on Halloween morning. Both kids were very dramatic about the whole thing, wailing about the indignity and injustice of it until I said, "Hey, that lady is dressed in a pilot uniform. And she is offering you stickers." End tears, return to school.





We enjoyed a week of relative good health here before a new cold hit the dynamic duo on Friday into Saturday. Now we're back to having our house sound like some kind of sanatorium, and poor Lime has coughed himself so hoarse that he sounds like a little frog. Lemon's voice is unaffected but he too has been coughing up a storm, with all the attendant setbacks in terms of nutrition. At this point I feel like if we just hold our ground we'll be winning--the possibility of exceeding his June weight any time soon seems remote. The good news is that in spite of having this pretty bad virus both kids are in good spirits, enjoying the kittens, and generally having a good time. The bad news is that we've had to miss food school again, the third time in 6 weeks. I am really wondering whether it's even worth continuing to pursue it in the winter, or whether we'd be better served by just waiting for spring again. Winter is just not an eating season for Lemon.

We got an upsetting letter in the mail this week: as of January 1, our insurance will no longer cover the pharmacy that we have been using since we moved here (formerly Target, now CVS within Target). When you fill as many prescriptions per month as we do (see illustrative photo), the relationship with the pharmacy is an important one, and we'd invested 4 years in this one, figuring out who the good and bad pharmacists were, getting the "auto" refill system to work the way we wanted, negotiating the copay cards and specialty pharmacy and all the rest. We're switching to the pharmacy at our children's hospital, on the theory that perhaps a pharmacy affiliated with a hospital might be able to handle a complex prescription load. Well, that and the fact that they aren't Walgreens, the other choice now offered by our insurance.

Another loss this week: the cart that we've used for Lemon's vest and associated equipment since we started on the vest reached the end of its service life this week. The kids and I had a good time putting together its replacement (thank you, Amazon, for remembering exactly what we ordered last time). It's hard to believe that we are already two years into using the vest. I recently ordered Lemon the next size up on the vest itself, since he'd outgrown the old one. Being aware of his deeply conservative nature, I ordered him the exact same color, just in a bigger size. I thought he'd be excited when the new one came, but instead he refused to even try it on. He was only willing to try it on when I promised that it was just a test, and that he would still use the old one. He did not anticipate that I would be so sneaky as to swap the old one out for the indistinguishable new one when he wasn't looking. The depths that parenting pushes you to...





Monday, October 30, 2017

Week 219: Bandit

Sometimes when I sit down to write the blog, I leaf through the photos from the week to remind myself of what happened. A thoughtful review of this week's photos indicates that we basically played with kittens non-stop. Not a bad way to spend a week, when you get down to it. We had been keeping the kittens downstairs in our office, but this weekend we decided that arrangement was too inconvenient and that they were sufficiently oriented to their new living situation that they could have the run of the house. Poor Daphne is still not totally on board with this new development, particularly since Donut likes to take a swipe at her with his paw, or bite her tail when it twitches in his vicinity. But, she is coming around and is definitely not unduly stressed. Our plan of getting two young cats seems to have paid off in that they mostly play with each other and leave her alone. Mostly.

















Today marks the end of our third week of Cayston, and with Lemon back to baseline we're going to stop treatment now and see how he does. Given that it's only October I'm sure we'll have many weeks in the not-so-distant future to do three treatments a day, so if we can take a break from that grind for a few weeks now I'd just as soon do it. We've been continuing to hammer away at nutrition, and he's finally back up over 38lb, ie, we're once again where we were in June. With any luck maybe we'll get up to 39lb before the next cold hits.






















We did have one "interesting" issue this week, which is that starting in the latter half of the week, we started noticing the occasional appearance of puddles around the house. They were too big to be kitten-related, and at first we thought they might be related to our new refrigerator with its tempting water dispenser and the imprudent use of open-topped cups, but they kept showing up in places where there were no cups, and far from the refrigerator. We observed, gathered clues, interviewed witnesses and suspects. All of which led to one conclusion: Lemon had been intentionally peeing at random locations around the house. Seriously? What is this? I was on board to do diapers and potty training and accidents. But intentionally peeing around the house? In his brother's bed? Nope. He seems like pretty much his usual self otherwise, and most of the time still uses the bathroom like a regular person. So what the heck? At the moment our only strategy is to positively reinforce the potty and hope that this behavior goes back to from whence it came, but any suggestions would be welcome!








Monday, October 23, 2017

Week 218: The scamper




I am pleased to report that somehow, we survived a week without Papa Bear. By somehow, I mainly mean that I did nothing but work and take care of the kids and went to bed at 8:30 every night. I may also have eaten cereal for dinner every night, it's kind of a blur at this point. But, every treatment got done, every diaper got changed, and the trash got taken out. So, victory.

We did do one extra fun thing this week, just to keep things interesting. As long-time readers will
recall, we originally had two cats, Daphne and Denzel. Sadly, Denzel passed away about 18 months ago, and my life has just not been complete without an orange boy cat in it. For whatever reason (sleep deprivation?) I thought that this would be a good time to bring a couple of little scamps into our household, so on Wednesday I picked up two little rescue kittens to come join the circus here in our house. The kids and Papa Bear had picked out the name Donut for one of the cats, so naturally I decided the other one (my sweet orange boy) should be named Duncan. They are pure joy. And Lemon is so in love with them. I suspect they will grow up to be VERY tolerant cats, since he will, without warning, scoop them up by their mid-sections and tote them around, put them down his little slide, have them "jump" on his trampoline. Daphne is still getting used to the idea that there are some new residents in the house, but I imagine she'll come around eventually. She did eventually accept the kids, after all!

In other news, some of you may remember that a while ago I finally managed to worm my way into the Cystic Fibrosis Foundation to do some medical writing for them. I'm very pleased to announce that some of my content has now made it onto their website. Feeling the need to brush up on some basics about the CFTR protein? Maybe you're interested the different types of CFTR mutations? How about CFTR modulators? Check out these all-new sections of CFF's website. I'm glad to say that the Foundation has asked me to keep going and develop some content for them about "New Frontiers in CF Research," so watch this space.








On the health front, we are done with two weeks of Cayston. If you had asked me a couple of days ago, I would have told you that we would be stopping Cayston after 2 weeks, because Lemon seemed really well. Unfortunately, Lemon, Lime, and I all caught some other new cold, hard on the heels of whatever the kids had before. So, Lemon is once again coughing up a storm. I'm quite convinced that it's just the new virus and not anything bacterial, but it seems pretty dumb to take him off the antibiotic when he's actively sick. After making up a little bit of precious ground in the past week, we're once again cutting back on tube feeds, dealing with puking, etc. This does make me feel better about all the boluses I pumped into him last week when he was well, though--we need those precious ounces of body fat to help him fight off this new cold, and hopefully we'll only get pushed back to where we started from, weight-wise, and not further into the hole.

We did cross an important milestone this week: it has now been one full year since Lemon was discharged from his most recent hospitalization. We are definitely in a stronger position than we were a year a go, and we've learned some important lessons about managing Lemon's health that have helped us to achieve our hospital-free year. I think the key lessons for us have been the importance of nutrition, hydration, and inhaled antibiotics. A year ago, we were so focused on the respiratory symptoms whenever Lemon got sick, and were not as attuned to the nutrition/hydration angle. Last year's hospitalization really showed us what a difference even just staying hydrated during an illness can make for him. We also got away from our long-standing intervention plan of oral antibiotics, in favor of inhaled. I won't lie, inhaled antibiotics are a pain. Three treatments a day gets old for everyone really fast. I long for the simplicity of shoving a few mL of a liquid medicine through a G-tube twice a day. But, much as I long for it, that plan never worked for us. And if inhaled antibiotics ever seem like a big deal, I just remind myself what doing home IV's is like, and then I remember that inhaled isn't so bad. Whatever this winter brings, we are more prepared to face it than ever before, and this hospital-free year is an accomplishment that we can all be proud of.

Monday, October 16, 2017

Week 217: Off to the races

Full speed ahead as always around here. On Monday night, within just a couple of hours of my posting the blog, Lemon's cough took a marked turn for the worse, so we decided we had no choice but to start Cayston on Tuesday morning. It was definitely the right call, since he started perking up almost immediately, and I'm hoping that we'll be able to get away with a two-week course instead of a month (knock on wood). Fitting in 3 treatments a day with school and work and everything else is challenging to say the least. It will definitely be a relief for everyone to get back to twice a day.
On Friday, Lemon had his follow-up with the gastroenterologist to talk about his liver. The first thing we learned at that appointment is that Lemon has lost a pound and a half relative to his weight in JUNE. That's 4 months of not gaining a thing and instead losing ground. Partly because of when we tried cutting back on tube feeds when we began food school, and partly from him being at least somewhat sick pretty much non-stop since school started, which forces us to keep the overnight feed volumes low. Now that we have this latest cold more or less under control, we are in full-court-press mode to try and get weight back on. That means 3.5 cartons of formula at night plus 100mL bolus feeds morning and night. It's a lot of formula, syringes, and fuss, but we've got to make up some ground while we can, since winter hasn't even started yet.

The next thing we learned from the GI doc was that he wanted us to start on a new medication, usrodiol, that may slow down or prevent damage to Lemon's liver. We started the ursodiol today, and we'll re-run the liver panel when we do his annual labs in December to see if it's having any effect. It better be, because a one month supply costs us $90 out of pocket (for a generic medication!). 

When we got back from the appointment, I dashed over to our local Enterprise and picked up a rental car, and on Saturday morning I drove down to Des Moines, where I met up with an old buddy from Boston. We bummed around Des Moines on Saturday afternoon and were very favorably impressed by it. On Sunday, we ran the Des Moines marathon together, capping off the 2017 running season with a personal best for me, 4:05. I didn't quite manage to break the 4:00 barrier this time around, but I think it was by far the strongest marathon I've ever run, and I have high hopes for the 2018 season. In the mean time, I'm looking forward to the off season, where I can run a little less and spend a little more time on other pursuits. Many thanks to Papa Bear, Nona, and the rest of our crew for taking such good care of the maniacs while I was away!
I'll definitely kick off the off-season in style this week, because no sooner did I return from Des Moines this morning then Papa Bear took off for a week in Greece for a conference and some sight-seeing. I'm sure I'll be doing plenty of running around over the next week, although most of it will be chasing after the dream team, rather than endless laps of the neighborhoods of Madison!

Monday, October 9, 2017

Week 216: Mountain high

This week, I briefly escaped the midwest for a work trip to Denver. You will all be pleased to know (not that I had any doubt) that the boys managed to survive on their own without me for a few days. Papa Bear did an admirable job holding down the fort by himself, including carrying out 3 treatments a day for Lemon, who (along with Lime) has had quite a cough for the past 10 days or so. I, meanwhile, had two of the most amazing nights of sleep that I've had within recent memory, waking up not at all during the night and sleeping until the deluxe hour of 5 a.m. mountain time (aka 6 a.m. central time) every morning. I think some work was done as well, but I mainly remember those nights of sleep. Amazing stuff, I tell you.









We have been going back and forth all week about whether to start Lemon on a round of Cayston. He has had this pretty terrible sounding productive cough for over a week, after all--a fresh cold of some kind after he kicked the first cold of the school year so well. Fortunately, we have Lime to serve as our canary in the coal mine. Our thinking is that if Lime is also still coughing, and if both of them still seem relatively perky, there is no reason to leap to the conclusion that there is something bacterial going on in Lemon's lungs. So far, we haven't seen any of the other signs (puking, fever, very low energy) that we typically see when Lemon needs a round of antibiotics, and we're continuing to wait. It seemed like both kids went through the worst of it a couple of days ago, and have both been getting incrementally better the last 48 hours or so. Fingers crossed. We could really use a break from all these colds to work on some weight gain!

Progress with eating continues to be, shall we say, mixed. Lemon's therapist at food school continues to be very enthusiastic about his progress, which I suppose is her job. I have to say that we are not seeing overwhelming progress. I think we are in a marginally better position than we were at the beginning of the summer. But, we're still not anywhere close to my seemingly conservative goal of getting Lemon to eat say 200 calories a day by mouth. Today, I asked the therapist what she thought of one of our other goals, which is getting Lemon to swallow pills. I caught what I believe is called a "microexpression" that rushed across her face before she composed herself and said something about how that was an interesting idea. I think we're going to try anyway. I had been holding back because I was hoping that Lemon would make real inroads on learning how to eat, but I feel like as long as we're going to sit in a plateau with regard to that skill, maybe he could at least try to learn a different oral skill in  the meantime. The nutritionist at our clinic was very supportive of the idea, and I think it would be helpful to Lemon (and to us!) in the long term, because he's started to fight us on taking his enzymes in applesauce, so we really need another approach.

All that aside, we had a fun time celebrating my birthday yesterday. Nona made some delicious cakes, and we went on our annual apple and pumpkin picking adventure. I also got a great birthday gift, a steam mop/vacuum. It probably says something about my advanced age (or the number of people in my house who throw/drop food on the floor every day, either preceded or followed by rolling in the sandbox and then running into the house) that this is one of the greatest birthday gifts ever. Thanks to everyone who sent birthday wishes from near and far, it was wonderful to hear from all of you!







Monday, October 2, 2017

Week 215: In the cold

It is so hard to sit down and write a blog post on a night like this, when, to top off all the other various crises that are going on in the world right now, a horrific mass shooting has taken place in Las Vegas. The infinitesimally small daily struggles of our household barely feel worth writing about. Honestly, they become hard for me to concentrate on, never mind all of you out there on the internet who aren't daily participants in these little dramas. And yet, despite the demands on my attention from the outside world, I know that I can't take my eye off the ball here even for a moment, because the nature of CF is such that if you coast for even a day, you will pay the price. So, I once again acknowledge that I am writing this blog as a reasonably well-informed citizen of our country and of the world, but that I will remain true to its focus on the life of one family living with CF.







We had a clinic visit on Wednesday, which went well all things considered. Lemon did very well with his pulmonary function tests. He was able to produce consistent results this time, and was much better at controlling his breath as he blew into the machine. He just needs to figure out how to breathe out for longer, and then we will be able to get some real numbers on how his lungs are doing. Of course, the fact that we have to go to the pulmonary lab at the beginning of every visit just makes these check-ups even longer. I had to suppress a laugh when our nurse practitioner (who we didn't even see until we'd been in the clinic for over two hours) told us that our _next_ visit is going to be "epic" because at that one we'll have to do a chest x-ray and a blood draw also. Trust me, two hours in an exam room with Lemon is already a fairly epic experience.





We are still struggling with weight gain. We'd backed down on tube feeding over the summer in order to give Lemon space to be hungry, to maximize the chances that food school would actually get him to start eating things by mouth. Instead he lost weight, so eventually in late July or early August we decided to go back up to his usual tube feeding volume. Then school started and he got a cold, so we had to back down on the volume again to avoid all the vomiting. Then he managed to get over that cold without any antibiotics (!!!!) so we went back up on the volume. That got us to Wednesday, where we'd managed to get his weight back up to just under where he was in June. Then, of course, having been well for a week or so, both kids came down with a second cold, so now we're back down on volume again. Sigh.






On the plus side, we once again have a working refrigerator, which feels like nothing short of a miracle. Lemon is fairly obsessed with the fact that water and ice come out of the front of it. And, one has to imagine that some day, somehow, he will become as interested in its contents as his younger brother is.

I'm leaving on a quick trip to Denver tomorrow morning, so we shall see how the males of the household do for 72 hours unsupervised. Dominoes Pizza may be called upon. Luckily Lime is now sleeping as late as 4:45 on a regular basis, so Papa Bear should be just fine. By which I mean, send coffee and forgive him if he seems sort of dazed. He should be back to normal operating parameters again by Monday I would think.